title: Pentalogy of Cantrell: Forty-two Years of Experience in the Hospital Infantil de Mexico Federico Gomez.
abstract: Pentalogy of Cantrell is a rare disease. Approximately 185 cases have been reported around the world. The authors performed a retrospective study that reviewed the clinical files and pathological samples of 22 cases of pentalogy of Cantrell treated at the Hospital Infantil de México Federico Gómez. Thirteen patients had ectopia cordis associated with pentalogy of Cantrell (group I), and there were 9 cases without ectopia cordis (group II). In group I, the following types of congenital heart disease were found: single ventricle (4), double-outlet right ventricle (4), ventricular septal defect (3), aortic coarctation (1), and atrial septal defect (1). In group II, the following types of congenital heart disease were found: double-outlet right ventricle (3), double-inlet left ventricle (2), ventricular septal defect (2), tetralogy of Fallot (1), and hypoplastic right ventricle syndrome (1). Nine cases had a ventricular diverticulum (40%). Ten patients (45%) had some other congenital anomaly associated with pentalogy of Cantrell. Thirteen patients underwent surgery (59%), which included cardiac surgery in 10 cases (45%). Sixteen patients died (73%): 11 from group I and 5 from group II (P < .05). Little more than 50 years since it was first described, pentalogy of Cantrell remains a disease with high mortality, especially in patients with associated ectopia cordis.
authors.affiliation: Departamento de Cardiología, Hospital Infantil de México Federico Gómez, México, D.F.
authors.forename: Norma
authors.initials: N
authors.lastname: Balderrábano-Saucedo
authors.forename: Alfredo
authors.initials: A
authors.lastname: Vizcaíno-Alarcón
authors.forename: Erika
authors.initials: E
authors.lastname: Sandoval-Serrano
authors.forename: Begoña
authors.initials: B
authors.lastname: Segura-Stanford
authors.forename: Luis A
authors.initials: LA
authors.lastname: Arévalo-Salas
authors.forename: Lorenzo Reyes
authors.initials: LR
authors.lastname: de la Cruz
authors.forename: Gonzalo
authors.initials: G
authors.lastname: Espinosa-Islas
authors.forename: Francisco Javier
authors.initials: FJ
authors.lastname: Puga-Muñuzuri
keywords: cardiac surgery; congenital heart disease; ectopia cordis; omphalocele; pentalogy of Cantrell; ventricular diverticulum